Short CommunicationSuccessful combined targeting of B- and plasma cells in treatment refractory anti-NMDAR encephalitis

- Targeting B- and plasma cells can be effective in refractory anti-NMDAR encephalitis.
- Neuronal cell loss is relatively limited in anti-NMDAR encephalitis.
- Brain atrophy can be reversible in anti-NMDAR encephalitis.
- This may explain the good long-term prognosis in those surviving the acute phase.

We describe an extremely severe case of therapy refractory NMDA receptor encephalitis (NMDAe) in a 26-year-old woman. After rituximab, bilateral oophorectomy, repeated cycles of high dose methylprednisolone and plasma exchange, she received repeated cyclophosphamide, tocilizumab (interleukin-6 inhibitor) and finally bortezomib (plasma cell depleting drug) leading to remission after 204 days in intensive care. Two years after disease onset her cognitive functions are still affected, but slowly improving and the cerebral atrophy has been partly reversed. The cerebrospinal fluid biomarker profile suggests an early synaptic/dendritic process, with subsequent neuroaxonal degeneration motivating aggressive treatment early on.

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